Courtney’s Spirit is Taking Action – What is PML?

What is PML…????

Progressive Multifocal Leuko Encephalopathy.

Courtney Leigh Hewitt

PML can also occur in organ transplant patients, people undergoing chronic corticosteroid or immunosuppressive therapy, and people that have certain cancers, such as Hodgkin’s disease, lymphoma, and sarcoidosis. PML disease most commonly occurs in anyone who has ever had the Chickenpox or has a weakened or compromised Immune System and affects the central nervous system and brain. The symptoms can be similar to other diseases out there, which is why it is so hard to detect.

The virus will cause lesions on the brain and begin to affect the nervous system and affects everyone differently. It depends on which section of the brain it decides to affect that will cause certain symptoms. For instance, when it attacks the part of the brain that controls a person’s motor skills that person will lose the ability to walk or talk. Early symptoms may include a weakness on one side, blurred vision, fatigue, impairment of learned skills, confusion, disorientation, loss of balance, and a possibility of seizures. Symptoms can often be misdiagnosed as a stroke or other similar HIV related conditions that affect the brain.

The only effective way of properly diagnosing PML Disease is through an MRI and CT Scan or a brain biopsy. The treatment of PML can be just as bad as retrieving the diagnosis. The treatment of medications was given through a shunt in the brain. Now it has been discovered that an aggressive anti-HIV therapy can possibly result in the remission of PML. If a person is misdiagnosed and does not receive the proper treatment, then their life expectancy will be one to three months.

Because this disease is so rare and little is known about it more people have passed away than should have.

Single mothers raising their children, such as Courtney Leigh Hewitt of Orlando, Florida have been misdiagnosed and did not receive the proper treatment. You can read Courtney’s Story in a new tell all book written by her loving father, Reginald Todd Hewitt.

The book tells of all the problems they faced in getting a proper diagnosis and the right treatment for Courtney. There are doctors, including HIV doctors, out there that do not know about PML, such as was the case with her own Infectious Disease Specialist!

PML is so rare and because of this many people are losing their lives when they could be living longer with the early diagnosis and aggressive treatments!

I hope that as an individual whether you have HIV, cancer, have had an organ transplant or know someone who has or is suffering from any of these diseases, you will educate yourself about this disease and read,” Courtney’s Story!

When you put the three parts together “leukoencephalopathy“ means a disease of the white matter in the brain.

But what does that mean?

Simply put, PML is a serious progressive viral opportunistic infection of the brain’s white matter. The virus responsible for PML is one that most everyone has been exposed to if they have ever had the Chickenpox!

PML itself is caused from the JC Virus, which is lying dormant in 85 to 90% of our World’s Population!!

According to information released on October 29, 2009 by Biogen Idec, there have been 24 confirmed cases of ‘progressive multifocal leukoencephalopathy’ (PML, a viral infection of the brain that usually leads to death or severe disability) among people who have used Tysabri® (natalizumab, Biogen Idec and Elan Pharmaceuticals) after it became available for prescription in July 2006. * As of the end of September 2009, 60,700 people have used Tysabri worldwide.

Although the absolute risk for PML in patients treated with Tysabri cannot be precisely determined, the sponsor has now released data suggesting that the risk increases with increasing time on therapy, starting out lower than the one-in-one thousand level that was estimated at the time of Tysabris re-approval in 2006, and rising after two years of infusions to about one in one thousand.

There is insufficient information to determine the risk of PML in those who have been on therapy for three years or more. Right now only 2,000 people have been on the therapy for over three years.

This release followed an October 23 announcement from the EMEA, the European equivalent of the U.S. FDA, indicating that one of its advisory committees was launching a review of the risks and benefits of Tysabri in light of the increasing number of new cases of PML.

Signs of PML:

Typical symptoms associated with PML progress quickly over days to weeks, and can include:

• personality or behavioral changes
• changes in thinking, memory, and orientation leading to confusion
• onset of seizures, clumsiness or progressive weakness on one side of the body
• disturbances of vision

If individuals taking Tysabri experience new, unusual symptoms, they should contact their prescribing physician immediately. Physicians who need guidelines on the protocol to follow when they have a patient on Tysabri who experiences unusual symptoms should contact Biogen Idec.

Details of Cases: According to the company, the 24 cases of PML have occurred in both men and women who had been given infusions of Tysabri every four weeks for a duration ranging from one year to three and a half years, with an average of two years.

·16 of the cases occurred in Europe, and 8 in the United States
·4 of the 24 died
·The degree of disability in the 20 survivors is a wide spectrum: at the milder end, some have recovered enough to return to work, and at the other extreme, some are confined to bed, requiring extensive assistance with activities of daily living, and others were in between this range. Further details of their condition were not provided.
·It appears that when PML is detected and treated early, it generally improves outcomes. It is important that individuals taking this drug and their doctors be vigilant in monitoring for any occurrence of new, unusual symptoms that might indicate PML.
·Based on these cases, the sponsor stressed that, contrary to prior information, the presence of gadolinium-enhancing lesions on MRI does not exclude the possibility of PML. Likewise, the absence of JC virus DNA in the spinal fluid does not exclude PML.
·There has been no characteristic among those who have developed PML that would give substantial clues to who might be more likely to develop it, except that half of the cases had prior histories of having been on immunosuppresive therapies, such as mitoxantrone, and less commonly, azathioprine and methotrexate.
·Right now there is no test that can predict who is more likely at risk for developing PML while using Tysabri; in a large company-sponsored study, testing of blood cells, plasma, serum and urine for the causative JC virus in people before and after 48 weeks of Tysabri therapy (Rudick et al, ECTRIMS 2009) did not show any differences in the presence of the virus in those fluids. The results of these studies, performed at the U.S. National Instituties of Health, differ somewhat from an earlier study (N. Engl. J. Med. 361:1067, 2009) suggesting higher virus levels after treatment.

·When PML was suspected, Tysabri infusions were halted. There is no specific therapy to treat PML, but the best hope is to reconstitute a persons immune responses. In most of the 24 cases, once PML was confirmed, Tysabri was removed from their systems with the blood-cleansing treatments of either plasma exchange or immunoadsorption.

During the aftermath of PML, as the immune system begins to recover, a condition called IRIS (immune reconstitution inflammatory syndrome) usually occurs about 4 weeks after the removal of Tysabri from the system. The sponsors suggested that some of the treating physicians found that prompt use of intravenous steroids to treat this brain inflammation led to improvement.

Since my daughter Courtney’s death, it has become my personal quest to Raise Global Awareness of this horrible virus!I would like to share her amazing story with you and your viewers around the world in hopes of educating and raising this much needed awareness.

My daughter, Courtney Leigh Hewitt, died last year of a rare viral disease called PML…..so rare her doctor had never even heard of it. He called himself an Infectious Disease Specialist. Holding the report in his hand, turns her away……..only to die a short time after……

Comments

hello, my 19 year old son was diagnosed with AIDS and PML on 10/25/11. He has been on and off of his medication since. We’ve been blessed with the news that it is actually getting better and his CD4 count is rising. However, he walks with a limp and stumbles a lot. I try to read articles about PML and came across yours. I’m sorry for your loss. Thank you for doing this, though.

So sorry for your loss of Courtney. I came across this article in my search for any clinical studies being held for PML. My son Brian, has been diagnosed with this awful disease. Originally diagnosed in Oct 2012 at VCU. He is now with his father in Clearwater, Fl and under doctors care from the HIV clinic. He also has a neurologist who I believe affiliated with the Mayo clinic at USF in Tampa. Brian has come a long way since being diagnosed and physically doing terrific. We just received the results of his MRI and the disease is in every part of the brain. The MRI taken at VCU was not clear enough to show any comparison, so our doctor will be doing another one in 3 months. Brian’s ‘C’ count was at a 39 in VA, and then a 177 in Dec. We will be getting the results of his latest blood test on Wednesday. They are aggressively treating Brian with SEVERAL medications which appear to be working quite well. Brian has short term memory loss and wets the bed every night. However he appears to be doing a lot better with other motor skills. If you know of any studies going on related to PML, please let me know. Prayers are with you and your family. Karen

Chantel,
Thank you for your comment. I would love to keep in touch and follow your mother’s progress. Please visit our site and I look forward to hearing from you. Prayers are with you on this journey!